The Heroic Chamber – an Outlook on the Right Ventricle in Eisenmenger Syndrome

Hernic, Alecsandra; Enache, Roxana; Radu, Daniela-Noela; Coman, Ioan M.; Ginghină, Carmen

doi:10.47803/rjc.2020.31.4.837

Figures & Tables

Comparison of echocardiographic characteristics in Eisenmenger syndrome secondary to a non-restrictive ventricular septal defect (left column) versus idiopathic pulmonary arterial hypertension (right column) RV, right ventricle. 1A. Transthoracic echocardiography, apical 4-chamber view, 2D examination: adaptive RV hypertrophy with no dilation. 1B. Transthoracic echocardiography, apical 4-chamber view, 2D examination: maladaptive RV hypertrophy and significant dilation. 1C. Transthoracic echocardiography, short-axis view, M-mode examination: adaptive RV hypertrophy with no dilation. 1D. Transthoracic echocardiography, short-axis view, M-mode examination: maladaptive RV hypertrophy and significant dilation. 1E. Transthoracic echocardiography, apical RV-focused view, M-mode examination: normal RV longitudinal function (TAPSE 20mm). 1F. Transthoracic echocardiography, apical RV-focused view, M-mode examination: RV longitudinal dysfunction (TAPSE 15mm). 1G. Transthoracic echocardiography, apical RV-focused view, TDI: normal RV longitudinal function (S’VD 13 cm/s). 1H. Transthoracic echocardiography, apical RV-focused view, TDI: RV longitudinal dysfunction (S’VD 9.5 cm/s). 1I. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view: mildly reduced RV systolic function (6-segments longitudinal strain −18.7%). 1J. Transthoracic 2D speckle-tracking echocardiography, apical RV-focused view – severe RV systolic dysfunction (6-segments longitudinal strain −8.2%)

Diagnostic work-up in Eisenmenger Syndrome (adapted from [4])

Clinical evaluation	SymptomsShortness of breath at rest/on exertionLimited exercise capacityPalpitationsHaemoptysisAnginaHeadacheDizziness Syncope/presyncope	Physical examinationWeightResting SpO₂%Blood pressureCyanosisDigital clubbingHeart rate, arrhythmiaSystemic congestion: oedema, jugular vein distension, hepatomegaly
ECG	Presence of sinus rhythmHeart rateSupraventricular/ventricular arrhythmiasConduction abnormalities (right bundle branch block/atrioventricular block)RV/biventricular hypertrophy	Holter monitoring may be considered in case of syncope, palpitations, baseline ECG abnormalities
Non-invasive imaging	Chest X-ray	Position of cardiac apex (RV hypertrophy)Cardiothoracic ratioPosition of aortic arch (left/right)Pulmonary outflow tract dilation/calcificationDilation of pulmonary arteriesPruning of peripheral pulmonary vesselsPleural/pericardial effusion
	TTE	Systematic analysis of cardiac morphology and ventriculo-arterial connectionsDescription of the shunt (location, direction, haemodynamic significance)RV and LV dimensions, systolic and diastolic functionLV eccentricity indexRA dimensions and areaPresence of pericardial effusionEstimation of PAP and RVEDP
	TEE (unanswered questions on TTE)	Shunt descriptionVentricular functionCo-existing valve diseaseCo-existing morphologic anomalies (i.e. anomalous pulmonary venous drainage)Suspicion of complications (intracardiac thrombosis/endocarditis)
	CMR (complex lesions/inadequate patient echogenicity)	Detailed description of cardiac morphologyShunt description and quantificationQuantification of RV volumes and RVEFRV fibrosis (LGE)
Non-invasive imaging	CT (specific indications)	Pulmonary artery diameters/calcificationPulmonary artery in situ thrombosisCompression of left main stem in case of pulmonary artery aneurysmSource of haemoptysis
Exercise testing	6MWD	Systematic at baseline and follow-up visits
Exercise testing	Cardiopulmonary exercise testing	Exercice capacityVO₂ max %
Cardiac catheterization	Confirmation of diagnosis and haemodynamic assessment–right atrial pressure, sPAP, mPAP, dPAP, capillary wedge pressure, pulmonary vascular resistances cardiac output, SVO₂ %, pulmonary-to-systemic flow ratio	Differential diagnosis: ES, PAH with left-to-right shunt, iPAH, segmental PH
Biomarkers	Full blood countRenal functionHepatic testsCoagulation panelNT-proBNPUric acidCRPSerum iron, ferritin, total iron binding capacity, transferrin saturation coefficientFolic acid, vitamin B12

The Heroic Chamber – an Outlook on the Right Ventricle in Eisenmenger Syndrome

Figures & Tables

Figure 1

Figure 2

Diagnostic work-up in Eisenmenger Syndrome (adapted from [4])

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